Ramsay Hunt syndrome (RHS) is a rare neurological disorder characterized by paralysis of the facial nerve (facial palsy) and a rash affecting the ear or mouth. Ear abnormalities such as ringing in the ears (tinnitus) and hearing loss may also be present. Ramsay Hunt syndrome is caused by the varicella zoster virus (VZV), the same virus that causes chickenpox in children and shingles (herpes zoster) in adults. In cases of Ramsay Hunt syndrome, previously inactive (dormant) varicella-zoster virus is reactivated and spreads to affect the facial nerve.
Several different names have been used to denote this disorder in the medical literature often causing confusion. The disorder is named after James Ramsay Hunt, a physician who first described the disorder in 1907. Year agos, more than one disorder bore the designation Ramsay Hunt syndrome. Ramsay-Hunt syndrome is now used to denote the disorder described in this report. The disorder is also sometimes known as herpes zoster oticus because of the characteristic ear rash. However, some physicians use herpes zostic oticus only for the ear rash and Ramsay Hunt syndrome for the combination of ear rash and facial paralysis.
Signs & Symptoms
The symptoms of Ramsay Hunt syndrome vary from case to case. Affected individuals usually experience paralysis (palsy) of the facial nerve and a rash affecting the ear. These two symptoms do not always occur simultaneously. In most cases, only one side of the face is affected (unilateral).
Facial muscles affected by nerve palsy may be weak or feel stiff and may result in the inability of affected individuals to smile, wrinkle the forehead or close their eye on the affected side. In some cases, speech may become slurred.
Most cases of Ramsay Hunt syndrome have a reddish (erythematous), painful, blistering (vesicular) rash that affects the outer portion of the ear (pinna) and often the external ear canal. In some cases, the rash, including painful blisters, may also affect the mouth, soft palate, and top portion of the throat. Some individuals with Ramsay Hunt syndrome may have facial palsy with evidence of varicella-zoster virus through testing (e.g., blood tests), but without the associated skin abnormalities. These cases may be referred to as zoster sine herpete.
Additional symptoms affecting the ear include ringing in the ear (tinnitus) and ear pain (otalgia). In some cases, ear pain may be intense. Pain may spread to affect the neck. Some affected individuals develop sensorineural hearing loss, a condition in which sound vibrations are not properly transmitted to the brain due to a defect of the inner ear or the auditory nerve, resulting in hearing loss. Hearing loss is usually temporary (transient), however, in rare cases it may become permanent.
In some cases, affected individuals may experience hyperacusis, a condition in which sounds appear louder (often dramatically) than normal. This can cause tremendous discomfort for affected individuals.
Additional symptoms that may be present include nausea, vomiting, and a sensation that one’s surroundings are spinning (vertigo). In rare cases, loss of taste, dry mouth, and dry eyes may also occur.
Ramsay Hunt syndrome is caused by the varicella-zoster virus, which is the same virus that causes chickenpox and shingles. The virus can remain dormant for decades in a person who has had chickenpox as a child. Reactivation of the varicella-zoster virus results in shingles and, in some cases, develops into Ramsay Hunt syndrome. The reason why the virus reactivates and affects the facial nerve in Ramsay Hunt syndrome is unknown.
Ramsay Hunt syndrome affects males and females in equal numbers. According to one estimate, 5 out of every 100,000 people develop Ramsay Hunt syndrome each year in the United States. The disorder is the second most common cause of atraumatic peripheral facial paralysis. Some researchers believe cases of Ramsay Hunt syndrome go undiagnosed or misdiagnosed making it difficult to determine the disorder’s true frequency in the general population.
Anyone who has had chickenpox can potentially develop Ramsay Hunt syndrome. However, most cases affect older adults, especially those over 60. Ramsay Hunt syndrome affecting children is extremely rare.
Symptoms of the following disorders can be similar to those of Ramsay Hunt syndrome.
Bell’s palsy is a nonprogressive neurological disorder of the facial nerve (7th cranial nerve). This disorder is characterized by the sudden onset of facial paralysis that may be preceded by a slight fever, pain behind the ear on the affected side, a stiff neck, and weakness and/or stiffness on one side of the face. Paralysis is thought to be caused by decreased blood supply (ischemia) and compression of the 7th cranial nerve. The exact etiology of Bell’s palsy is not known. Viral and immune disorders are frequently implicated as a cause for this disorder. There may also be an inherited tendency toward developing Bell’s palsy. (For more information on this disorder, choose “Bell’s palsy” as your search term in the Rare Disease Database.)
Acoustic neuroma is a benign tumor of the 8th cranial nerve. This nerve lies within the internal ear canal. Pressure on this nerve results in the early symptoms of acoustic neuroma; a ringing sound in the ear (tinnitus), and/or hearing loss may occur. An associated compression of the facial nerve (7th cranial nerve) may produce muscle weakness; pressure on the trigeminal nerve (5th cranial nerve) may lead to facial numbness. The expansion of the tumor into different areas may result in impaired ability to coordinate movement of the legs and arms (ataxia), numbness in the mouth, slurred speech (dysphagia), and/or hoarseness. (For more information in this disorder, choose “acoustic neuroma” as your search term on the Rare Disease Database.)
Trigeminal neuralgia, also known as tic douloureux, is a disorder of the 5th cranial nerve (trigeminal nerve) characterized by attacks of intense, stabbing pain affecting the mouth, cheek, nose, and/or other areas on one side of the face. The exact cause of trigeminal neuralgia is not fully understood. (For more information on this disorder, choose “trigeminal neuralgia” as your search term in the Rare Disease Database.)
A diagnosis of Ramsay Hunt syndrome is based on thorough clinical evaluation, a detailed patient history and identification of characteristic symptoms (i.e. facial palsy and rash). Viral studies can detect varicella-zoster virus in saliva, tears, and blood but are not necessary to establish a diagnosis of Ramsay Hunt syndrome.
The diagnosis of Ramsay Hunt syndrome can be difficult because the specific symptoms of the disorder (otalgia, facial paralysis and the distinctive rash) do not always develop at the same time.
The treatment of Ramsay Hunt syndrome commonly involves antiviral medications, like acyclovir or famciclovir, in conjunction with corticosteroids, like prednisone. Most authorities agree that beginning antiviral treatment within three days of onset appears to have the greatest benefit as prompt diagnosis and management seems to improve outcomes. Despite therapy, some degree of facial paralysis and hearing loss may become permanent in some cases.
Further treatment is directed towards specific symptoms that are apparent in each individual. This includes pain medication, carbamazepine, an anti-seizure medicine which may help reduce neuralgic pain, and vertigo suppressants like antihistamines and anticholinergics.
Individuals with Ramsay Hunt syndrome need to take special care to prevent corneal injury because the inability to properly close the eye can expose the cornea to abnormal drying and foreign body irritation. Artificial tears and lubricating ointments may be prescribed to protect the cornea.